ICD-10: C83.8

Other non-follicular lymphoma

Clinical Information

Inclusion Terms

  • Lymphoid granulomatosis
  • Primary effusion B-cell lymphoma
  • Intravascular large B-cell lymphoma

Additional Information

Description

ICD-10 code C83.8 refers to "Other non-follicular lymphoma," which is classified under the broader category of non-Hodgkin lymphoma (NHL). This code is used to identify specific types of lymphomas that do not fall under the more common follicular lymphoma classification. Below is a detailed overview of this condition, including its clinical description, characteristics, and relevant information.

Clinical Description

Definition

Non-follicular lymphoma encompasses a variety of lymphoproliferative disorders that originate from B-cells or T-cells but do not exhibit the follicular pattern typical of follicular lymphoma. The term "other" indicates that these lymphomas may have unique histological features or clinical presentations that distinguish them from more commonly recognized types of non-Hodgkin lymphoma.

Types of Non-Follicular Lymphoma

The category of "other non-follicular lymphoma" includes several subtypes, which may vary in their biological behavior, treatment response, and prognosis. Some of these subtypes include:

  • Diffuse Large B-Cell Lymphoma (DLBCL): This is the most common subtype of non-Hodgkin lymphoma and is characterized by large, rapidly growing tumors.
  • Mantle Cell Lymphoma: A more aggressive form of lymphoma that typically presents with advanced disease at diagnosis.
  • Burkitt Lymphoma: A highly aggressive lymphoma that is often associated with a specific chromosomal translocation.
  • Peripheral T-Cell Lymphoma: A diverse group of aggressive lymphomas that arise from T-cells.

Symptoms

Patients with non-follicular lymphoma may present with a variety of symptoms, which can include:

  • Swollen lymph nodes (lymphadenopathy)
  • Fever
  • Night sweats
  • Unexplained weight loss
  • Fatigue
  • Abdominal pain or swelling

These symptoms can vary significantly depending on the specific subtype of lymphoma and the extent of disease involvement.

Diagnosis and Staging

Diagnosis of non-follicular lymphoma typically involves a combination of clinical evaluation, imaging studies (such as CT scans or PET scans), and histopathological examination of lymph node biopsies. The staging of lymphoma is crucial for determining the appropriate treatment approach and prognosis. The Ann Arbor staging system is commonly used, which classifies the disease based on the number of lymph node regions involved and the presence of systemic symptoms.

Treatment Options

Treatment for non-follicular lymphoma varies based on the specific subtype, stage of the disease, and patient factors. Common treatment modalities include:

  • Chemotherapy: Often the first-line treatment, especially for aggressive forms of lymphoma.
  • Radiation Therapy: May be used in localized disease or as part of a combined modality approach.
  • Targeted Therapy: Newer treatments that target specific molecular pathways involved in lymphoma growth.
  • Stem Cell Transplantation: Considered for eligible patients with relapsed or refractory disease.

Prognosis

The prognosis for patients with non-follicular lymphoma can vary widely based on the specific subtype, stage at diagnosis, and response to treatment. Generally, aggressive forms of lymphoma may have a poorer prognosis, while indolent forms may have a more favorable outcome.

Conclusion

ICD-10 code C83.8 serves as a critical identifier for healthcare providers when diagnosing and treating patients with other non-follicular lymphoma. Understanding the various subtypes, symptoms, diagnostic methods, and treatment options is essential for effective management of this complex group of diseases. As research continues to evolve, new therapies and treatment strategies are being developed, offering hope for improved outcomes for patients diagnosed with non-follicular lymphoma.

Clinical Information

Non-follicular lymphoma, classified under ICD-10 code C83.8, encompasses a variety of lymphomas that do not fall into the follicular category. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.

Clinical Presentation

Overview of Non-Follicular Lymphoma

Non-follicular lymphomas are a subset of non-Hodgkin lymphomas (NHL) that include various histological types, such as diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma, and others. These lymphomas are characterized by their aggressive nature and can present in various ways depending on the specific subtype and the extent of disease.

Common Signs and Symptoms

Patients with non-follicular lymphoma may exhibit a range of signs and symptoms, which can vary based on the lymphoma subtype and the organs involved. Common manifestations include:

  • Lymphadenopathy: Swelling of lymph nodes, often painless, is one of the most common signs. This can occur in cervical, axillary, or inguinal regions.
  • B Symptoms: These systemic symptoms include fever, night sweats, and unexplained weight loss, which are indicative of more aggressive disease.
  • Fatigue: Patients often report significant fatigue, which can be debilitating and is a common complaint in lymphoma cases.
  • Abdominal Symptoms: If the lymphoma involves the abdomen, patients may experience abdominal pain, distension, or changes in bowel habits due to lymphadenopathy or organ involvement.
  • Skin Manifestations: Some patients may develop skin lesions or rashes, particularly in certain subtypes like cutaneous T-cell lymphoma.

Additional Symptoms

  • Respiratory Symptoms: If lymph nodes in the chest are involved, patients may experience cough, shortness of breath, or chest pain.
  • Neurological Symptoms: In cases where the central nervous system is affected, symptoms may include headaches, seizures, or neurological deficits.

Patient Characteristics

Demographics

  • Age: Non-follicular lymphomas can occur at any age but are more common in adults, particularly those over 60 years old.
  • Gender: There is a slight male predominance in many types of non-follicular lymphoma, although this can vary by specific subtype.

Risk Factors

  • Immunocompromised States: Patients with weakened immune systems, such as those with HIV/AIDS or those on immunosuppressive therapy, are at increased risk for developing non-follicular lymphomas.
  • Previous Cancers: Survivors of other malignancies, particularly breast cancer, have a documented increased risk of developing non-Hodgkin lymphoma, including non-follicular types[3][10].
  • Environmental Exposures: Certain environmental factors, such as exposure to pesticides or solvents, have been associated with an elevated risk of lymphoma.

Comorbidities

Patients with non-follicular lymphoma may also present with other health conditions, which can complicate treatment and management. Common comorbidities include cardiovascular diseases, diabetes, and other malignancies.

Conclusion

Non-follicular lymphoma, represented by ICD-10 code C83.8, presents with a diverse array of clinical features and patient characteristics. Recognizing the signs and symptoms, such as lymphadenopathy, systemic B symptoms, and fatigue, is essential for timely diagnosis and intervention. Understanding the demographics and risk factors associated with this condition can aid healthcare providers in identifying at-risk populations and tailoring appropriate management strategies. Early detection and treatment are critical for improving patient outcomes in this aggressive form of lymphoma.

Approximate Synonyms

ICD-10 code C83.8 refers to "Other non-follicular lymphoma," which encompasses a variety of lymphoproliferative disorders that do not fall under the more common categories of follicular lymphoma. Understanding the alternative names and related terms for this classification can be beneficial for healthcare professionals, researchers, and patients alike.

Alternative Names for C83.8

  1. Non-Follicular Lymphoma: This is a broad term that includes various types of lymphomas that do not exhibit follicular patterns.
  2. Other Types of Non-Hodgkin Lymphoma (NHL): C83.8 is often categorized under the umbrella of non-Hodgkin lymphomas, which are a diverse group of blood cancers that include all lymphomas except Hodgkin's lymphoma.
  3. B-cell Lymphoma: Many non-follicular lymphomas are of B-cell origin, and thus may be referred to in this context.
  4. Peripheral T-cell Lymphoma (PTCL): Some non-follicular lymphomas may be classified under this category, although PTCL has its own specific codes in the ICD-10 system.
  5. Lymphoproliferative Disorders: This term encompasses a range of conditions, including various types of lymphomas, characterized by the excessive production of lymphocytes.
  1. Intravascular Large B-cell Lymphoma (IVLBCL): A rare subtype of non-Hodgkin lymphoma that may be included under C83.8, characterized by the proliferation of large B-cells within blood vessels[6].
  2. Lymphomatoid Granulomatosis (LYG): A rare condition that can be associated with non-follicular lymphomas, particularly grade 3, which may also be coded under C83.8[8].
  3. Diffuse Large B-cell Lymphoma (DLBCL): While DLBCL has its own specific ICD-10 code, it is often discussed in relation to other non-follicular lymphomas due to its prevalence and characteristics.
  4. Mantle Cell Lymphoma: Another subtype of non-Hodgkin lymphoma that, while typically classified separately, shares some clinical features with other non-follicular lymphomas.

Conclusion

ICD-10 code C83.8 serves as a classification for various non-follicular lymphomas, which can be referred to by several alternative names and related terms. Understanding these terms is crucial for accurate diagnosis, treatment planning, and coding in medical records. For healthcare professionals, being familiar with these classifications can enhance communication and ensure comprehensive patient care.

Treatment Guidelines

When discussing the standard treatment approaches for ICD-10 code C83.8, which refers to "Other non-follicular lymphoma," it is essential to understand the broader context of non-Hodgkin lymphoma (NHL) and the specific characteristics of various subtypes. Non-follicular lymphomas encompass a range of aggressive and indolent lymphomas that do not fall under the follicular category, including types such as diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma, and others.

Overview of Non-Follicular Lymphoma

Non-follicular lymphomas are characterized by their diverse histological features and clinical behaviors. They can be aggressive or indolent, and their treatment often depends on the specific subtype, stage of the disease, and the patient's overall health. The most common subtype within this category is DLBCL, which is known for its aggressive nature and requires prompt treatment.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy remains a cornerstone of treatment for many non-follicular lymphomas. The most widely used regimen for aggressive forms, particularly DLBCL, is the R-CHOP regimen, which includes:

  • Rituximab: A monoclonal antibody targeting CD20 on B-cells.
  • Cyclophosphamide: An alkylating agent.
  • Doxorubicin (Adriamycin): An anthracycline antibiotic.
  • Vincristine: A vinca alkaloid.
  • Prednisone: A corticosteroid.

This combination is typically administered in cycles over several months, with the exact number of cycles depending on the response to treatment and the specific lymphoma subtype[1][2].

2. Targeted Therapy

In addition to traditional chemotherapy, targeted therapies have become increasingly important in the treatment of non-follicular lymphomas. For instance:

  • Bruton’s Tyrosine Kinase (BTK) Inhibitors: Such as ibrutinib, are used particularly in mantle cell lymphoma and other B-cell malignancies.
  • BCL-2 Inhibitors: Like venetoclax, are effective in certain subtypes, especially those with specific genetic mutations[3].

3. Radiation Therapy

Radiation therapy may be employed in localized cases or as a consolidation treatment after chemotherapy. It is particularly useful for patients with localized disease or those who have residual disease after initial treatment. The decision to use radiation therapy is based on the lymphoma's stage and the patient's overall treatment plan[4].

4. Stem Cell Transplantation

For patients with aggressive non-follicular lymphomas who do not respond adequately to initial therapy or who experience relapse, autologous stem cell transplantation may be considered. This approach involves harvesting the patient's stem cells, administering high-dose chemotherapy to eradicate the lymphoma, and then reinfusing the stem cells to restore bone marrow function[5].

5. Clinical Trials

Participation in clinical trials is encouraged for patients with non-follicular lymphomas, as these studies often provide access to novel therapies and treatment regimens that may offer improved outcomes compared to standard treatments. Clinical trials may focus on new chemotherapy agents, targeted therapies, or immunotherapies[6].

Conclusion

The treatment of non-follicular lymphoma (ICD-10 code C83.8) is multifaceted and tailored to the individual patient based on the specific subtype, disease stage, and overall health. Standard approaches typically include chemotherapy, targeted therapies, radiation, and potentially stem cell transplantation. As research continues to evolve, clinical trials remain a vital avenue for exploring new treatment options that may enhance patient outcomes. For patients diagnosed with this condition, a multidisciplinary approach involving oncologists, hematologists, and other specialists is crucial for optimizing care and improving survival rates.

Diagnostic Criteria

The diagnosis of non-follicular lymphoma, specifically under the ICD-10 code C83.8, involves a comprehensive evaluation that includes clinical, histopathological, and imaging criteria. Below is a detailed overview of the criteria used for diagnosing this condition.

Clinical Presentation

Symptoms

Patients with non-follicular lymphoma may present with a variety of symptoms, which can include:
- Lymphadenopathy: Swelling of lymph nodes, often painless.
- B Symptoms: These include fever, night sweats, and unexplained weight loss, which are indicative of systemic involvement.
- Fatigue: Generalized weakness or fatigue is common.
- Splenomegaly: Enlargement of the spleen may occur.

Medical History

A thorough medical history is essential, including:
- Previous history of lymphoproliferative disorders.
- Family history of lymphomas or other malignancies.
- Exposure to risk factors such as certain infections (e.g., Epstein-Barr virus) or environmental toxins.

Histopathological Criteria

Biopsy

A definitive diagnosis of non-follicular lymphoma requires a biopsy of the affected lymph node or tissue. The following histopathological features are evaluated:
- Cell Type: Identification of the specific type of lymphoma cells (e.g., diffuse large B-cell lymphoma, mantle cell lymphoma).
- Cellularity: Assessment of the density and arrangement of the lymphoma cells.
- Immunophenotyping: Use of immunohistochemical staining to determine the expression of specific markers (e.g., CD20, CD5) that help classify the lymphoma subtype.

Cytogenetic and Molecular Studies

  • Genetic Testing: Certain genetic abnormalities (e.g., translocations) can aid in diagnosis and classification.
  • Molecular Profiling: Techniques such as PCR may be used to detect specific genetic markers associated with non-follicular lymphomas.

Imaging Studies

Radiological Evaluation

Imaging studies are crucial for staging and assessing the extent of the disease:
- CT Scans: Computed tomography scans of the chest, abdomen, and pelvis to identify lymphadenopathy and organ involvement.
- PET Scans: Positron emission tomography can help assess metabolic activity of the lymphoma and detect distant spread.

Staging

The Ann Arbor staging system is commonly used to classify the extent of lymphoma:
- Stage I: Involvement of a single lymph node region.
- Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm.
- Stage III: Involvement of lymph node regions on both sides of the diaphragm.
- Stage IV: Disseminated involvement of one or more extralymphatic organs.

Conclusion

The diagnosis of non-follicular lymphoma (ICD-10 code C83.8) is a multifaceted process that combines clinical evaluation, histopathological examination, imaging studies, and staging. Accurate diagnosis is critical for determining the appropriate treatment strategy and improving patient outcomes. If you suspect non-follicular lymphoma, it is essential to consult a healthcare professional for a thorough assessment and diagnosis.

Related Information

Description

  • Non-follicular lymphomas originate from B-cells or T-cells
  • No follicular pattern typical of follicular lymphoma
  • Unique histological features or clinical presentations
  • Subtypes include DLBCL, Mantle Cell Lymphoma, Burkitt Lymphoma, Peripheral T-Cell Lymphoma
  • Symptoms include swollen lymph nodes, fever, night sweats, unexplained weight loss, fatigue, abdominal pain or swelling
  • Diagnosis involves clinical evaluation, imaging studies and histopathological examination of lymph node biopsies
  • Treatment varies based on subtype, stage and patient factors

Clinical Information

  • Lymphadenopathy: Swelling of lymph nodes
  • B Symptoms: Fever, night sweats, unexplained weight loss
  • Fatigue: Debilitating and common complaint
  • Abdominal Symptoms: Pain, distension, bowel changes
  • Skin Manifestations: Skin lesions or rashes
  • Respiratory Symptoms: Cough, shortness of breath, chest pain
  • Neurological Symptoms: Headaches, seizures, neurological deficits
  • Age: Common in adults over 60 years old
  • Gender: Slight male predominance
  • Immunocompromised States: Increased risk for weakened immune systems
  • Previous Cancers: Elevated risk after other malignancies
  • Environmental Exposures: Pesticides, solvents linked to lymphoma

Approximate Synonyms

  • Non-Follicular Lymphoma
  • Other Types of NHL
  • B-cell Lymphoma
  • Peripheral T-cell Lymphoma
  • Lymphoproliferative Disorders
  • Intravascular Large B-cell Lymphoma
  • Lymphomatoid Granulomatosis
  • Diffuse Large B-cell Lymphoma
  • Mantle Cell Lymphoma

Treatment Guidelines

  • Chemotherapy is cornerstone of treatment
  • R-CHOP regimen used for aggressive forms
  • Rituximab targets CD20 on B-cells
  • Cyclophosphamide is an alkylating agent
  • Doxorubicin is anthracycline antibiotic
  • Vincristine is vinca alkaloid
  • Prednisone is corticosteroid
  • Targeted therapy used for specific subtypes
  • Bruton’s Tyrosine Kinase Inhibitors are effective
  • BCL-2 Inhibitors used in certain subtypes
  • Radiation therapy used for localized disease
  • Stem cell transplantation considered for relapse
  • Clinical trials provide access to novel therapies

Diagnostic Criteria

Coding Guidelines

Excludes 1

  • T-cell rich B-cell lymphoma (C83.3-)
  • mediastinal (thymic) large B-cell lymphoma (C85.2-)

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